What is fibromuscular dysplasia (FMD)?

Updated: Jul 27, 2018
  • Author: James A Wilson, MD, MSc, FRCPC; Chief Editor: Helmi L Lutsep, MD  more...
  • Print
Answer

Fibromuscular dysplasia (FMD) was first observed in 1938 by Leadbetter and Burkland in a 5-year-old boy, and described as a disease of the renal arteries. Involvement of the craniocervical arteries was recognized in 1946 by Palubinskas and Ripley.

FMD is an angiopathy that affects medium-sized arteries predominantly in young women of childbearing age. FMD most commonly affects the renal arteries and can cause refractory renovascular hypertension. Of patients with identified FMD, renal involvement occurs in 60-75%, cerebrovascular involvement occurs in 25-30%, visceral involvement occurs in 9%, and arteries of the limbs are affected in about 5%. [1, 2] Case reports have shown FMD in most other medium-to-large arteries as well, including the coronary arteries [3] , the pulmonary arteries [4] , and the aorta [5] . In 26% of patients, disease is found in more than one arterial region [6] .

In patients with identified cephalic FMD, 95% have internal carotid artery involvement and 12-43% have vertebral artery involvement. Although FMD can affect arteries of any size [7] , involvement of smaller ones, including intracranial vessels, is rare. Although an early autopsy series of 819 consecutive patients found the prevalence of FMD in the internal carotid arteries to be 1% [8] , a larger, more recent autopsy series of 20,244 patients recently identified the overall prevalence of FMD of the internal carotid arteries to be only 0.02% [9] . From a neurologic perspective, FMD is an important cause of stroke in young adults.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!